What is the treatment for Churg-Strauss syndrome (Eosinophilic Granulomatosis with Polyangiitis, EGPA)?

From the Guidelines

Churg-Strauss syndrome, now called eosinophilic granulomatosis with polyangiitis (EGPA), is treated with a combination of medications tailored to disease severity, with the primary goal of inducing remission and maintaining it while minimizing treatment-related toxicity, as recommended by the most recent guidelines 1. The treatment approach is based on the severity of the disease, with severe cases requiring more aggressive therapy. For patients with severe EGPA, high-dose glucocorticoids plus cyclophosphamide or rituximab are recommended for remission induction, as supported by recent studies 1. In contrast, for patients with non-severe EGPA, mepolizumab and glucocorticoids are conditionally recommended over methotrexate, azathioprine, or mycophenolate mofetil and glucocorticoids, due to the efficacy of mepolizumab in a randomized trial 1. Some key points to consider in the treatment of EGPA include:

• The use of glucocorticoids as the cornerstone of therapy, with the dose and duration tailored to the individual patient's response and disease severity.
• The addition of immunosuppressants such as cyclophosphamide or rituximab in severe cases, or mepolizumab in non-severe cases, to induce and maintain remission.
• The importance of addressing underlying asthma and allergic symptoms with appropriate inhalers and antihistamines.
• Regular monitoring of blood counts, organ function, and disease activity, as well as pneumocystis pneumonia prophylaxis with trimethoprim-sulfamethoxazole when using immunosuppressants. Overall, the treatment of EGPA requires a comprehensive approach that takes into account the individual patient's disease severity, response to therapy, and potential treatment-related toxicity, with the goal of achieving and maintaining remission while minimizing morbidity and mortality, as emphasized by the latest evidence-based guidelines 1.

From the FDA Drug Label

1. 3 Eosinophilic Granulomatosis with Polyangiitis NUCALA is indicated for the treatment of adult patients with eosinophilic granulomatosis with polyangiitis (EGPA).

Treatment of Churg-Strauss (EGPA): Mepolizumab (NUCALA) is indicated for the treatment of adult patients with eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome.

• The recommended dosage is 300 mg administered subcutaneously once every 4 weeks 2.
• It is essential to note that the treatment should be used under the guidance of a healthcare professional, and the patient's condition should be closely monitored.
• Patients with EGPA should be on a stable dosage of oral prednisolone or prednisone of greater than or equal to 7.5 mg/day (but not greater than 50 mg/day) for at least 4 weeks prior to enrollment 2.

From the Research

Treatment Options for Churg-Strauss Syndrome

• Corticosteroids are the primary treatment for Churg-Strauss syndrome, with the goal of rapidly treating active vasculitis 3
• In patients with severe disease, the addition of cyclophosphamide may improve outcomes and reduce relapses 3, 4
• For patients with less severe disease, methotrexate can be used as a corticosteroid-sparing agent to reduce the cumulative dose of corticosteroids 3
• In very severe cases, anti-TNF blocking agents such as infliximab or etanercept may be added for a limited period of time 3, 4
• Recombinant IFN-alpha can also be effective in refractory cases when given on a short-term basis 3

Immunosuppressive Therapy

• Azathioprine and intravenous pulse cyclophosphamide can be used as adjuvant immunosuppressive therapy for treatment failure or relapse 5
• Mepolizumab, a humanized monoclonal anti-IL-5 antibody, can safely allow corticosteroid tapering in patients with steroid-dependent Churg-Strauss syndrome 6
• IVIG therapy may be a hopeful candidate for second-line treatment for patients with severe CSS, particularly in cases of neuropathy and/or cardiomyopathy 7

Treatment Goals and Outcomes

• The primary goal of treatment is to induce remission and prevent relapses 3, 7, 5
• Treatment outcomes can vary depending on the severity of the disease and the presence of poor-prognosis factors 7, 5
• Survival rates can be excellent in patients with Churg-Strauss syndrome, particularly in those without poor-prognosis factors 5